Data on epilepsy surgery in children originate mostly from small case series of up to 15 patients and reports of single cases. Patient selection in most case series required the diagnosis of medically refractory epilepsy and RSE not responding to HDST encompassing treatment with phenobarbitone, propofol, thiopental, and/or midazolam [6].

A comparative study of patients who underwent epilepsy surgery with and without RSE revealed that the six patients with RSE had an earlier seizure onset (mean of 1.7 years vs. 2.4 years in the non-RSE group) and underwent hemispherotomy more often than the non-RSE group (83% vs. 56%, respectively; [16]). All patients had identifiable structural MRI lesions (cortical dysplasia, Rasmussen encephalitis, and perinatal stroke). In all patients, RSE ceased after surgery and 75% of the patients were seizure free at the 2‑year follow-up.

In another survey of ten pediatric patients, RSE was terminated in all patients by surgery [15]. All of the patients required ICU care and nine needed mechanical ventilation prior to surgery. Eight of the ten patients had congruent EEG and MRI findings. The majority of patients (60%) underwent hemispherotomy. Rates of seizure freedom were reported in 70% of the case (the observation period ranged from 4 months to 6.5 years). The two patients with non-congruent EEG and MRI findings did not become seizure-free. Two out of the three patients who did not become seizure free significantly improved in their epilepsy compared with the preoperative state. There was significant presurgical morbidity, which was interpreted mainly as a result of HDST (blood pressure instability, requirement of inotropic support, pneumonia, urinary tract infection, line infection, Clostridium difficile colitis, fever of unknown origin, neutropenia, paralytic ileus, pneumothorax, and lower-extremity deep venous thrombosis).

A study of 15 patients undergoing epilepsy surgery due to RSE comprised a more heterogeneous selection of cases as almost one third of the patients did not have a clear focal structural pathology in brain imaging [10]. Consequently, congruence of EEG and MRI was poor in the majority of these patients. In all cases, RSE was ended by surgery (one patient needed re-surgery) and seizure freedom at follow-up (at least 14 months) was seen in seven patients (47%). Two out of the six patients harboring non-localizing EEG abnormalities became seizure free. Overall, 13 out of 15 patients had nuclear imaging (either an ictal SPECT or FDG-PET study) and the majority of patients had localized abnormalities, which was used to guide further intraoperative ECoG and subsequent resection.

The most recent survey reported on ten patients undergoing epilepsy surgery in the presence of RSE. Similar to previous results, patients were young at the time of seizure onset (mean of 2.7 years) and underwent hemispherotomy in the majority of cases (70%). All patient had focal MRI abnormalities. Ictal EEG and MRI concordance was 90% for correct lateralization. Seizure outcome was categorized as Engel Class Ia (completely seizure free since surgery) in 90% of the cases and also included the one patient with diffuse ictal EEG findings.

Further case reports documented noteworthy single-case experiences in special clinical constellations and included resective surgery in n-methyl aspartate receptor (NMDAR) encephalitis and a mitochondrial disorder, or other therapeutical surgical approaches such as deep brain stimulation (DBS) or corpus callosotomy (CC).

Two pediatric patients aged 9 and 5 years with febrile infection-related epilepsy syndrome (FIRES) and RSE requiring HDST underwent centromedian thalamic nuclei DBS and anakinra treatment and showed remarkably different outcomes. Clinically meaningful seizure reduction prompting ICU discharge and good long-term outcome (attending regular school besides some seizures) was reported for the 9‑year-old patient, whereas no relevant clinical improvement was observed in the other patient [18].

Another patient (9 years old) with FIRES and RSE who underwent centromedian thalamic nuclei DBS (without anakinra treatment) demonstrated considerable improvement in baseline mental status 30 days after DBS insertion and ICU discharge, although seizure freedom was not achieved [17].

Resective surgery was performed on a 7-year-old patient with refractory partial SE secondary to NMDAR antibody encephalitis [19]. Left occipital resection was performed after 3 months of HDST treatment on the basis of localized EEG seizure activity, ictal hyperperfusion (SPECT) and cortical hypometabolism (FDG-PET) in the left posterior region. Intraoperative ECoG also revealed left occipital ictal activity. Status epilepticus stopped immediately after surgery (left occipital lobectomy) and the patient was discharged from the ICU 12 days later. At follow-up after 2 years, homonymous hemianopia and dysphasia were present. The patient was able to eat on his own but needed supervision in almost all other activities. He was still on four ASM and suffered from two to three seizures per week.

A 4-year-old child underwent palliative hemispherotomy due to medically refractory epilepsia partialis continua (EPC) of the right hemisphere caused by a heterozygous POLG1 mutation (Alpers disease), which prompted some ethical concerns [20, 21]. She was ventilated due to involvement of the left diaphragm by the EPC. After right-sided hemispherotomy, EPC stopped and the patient was discharged from the ICU. She died 2 months later due to liver failure secondary to a respiratory infection.

A 9-year-old boy with RSE of unknown cause (no focal lesions were detected on high-resolution MRI, and both EEG and ictal SPECT were not lateralizing) requiring HDST underwent CC. The RSE stopped after CC. At 6 months after surgery, neurological function had recovered to the baseline before the RSE occurred [22]. Data on vagal nerve stimulation (VNS) in pediatric RSE are sparse. In a retrospective survey of 16 patients, the authors reported on a decreased risk of SE after VNS implantation [23]. However, the patients did not undergo implantation during the acute phase of SE after receiving HDST. Similar findings were provided by a systematic review of VNS in refractory and super-refractory status in both children and adults [24]. As the information about VNS regimes was inconsequent, the authors questioned whether reporting of the treatments covered the refractory phase only.